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The Hospital for Sick Children, Great Ormond Street, London, W.C.1
This excerpt was created in the absence of an abstract.
The morbid anatomical and radiological features of the Dandy-Walker syndrome are now well known mainly through the work of Dandy and Blackfan (1914) Taggert and Walker (1942), Benda (1954), Gibson (1955) and Matson (1956). Benda (1954) suggested the eponym Dandy-Walker syndrome, which usually implies agenesis of the cerebellar hemispheres, gross dilatation of the 4th ventricle, occlusion or absence of the foramen of Magendie, high position of the torcula herophili and lateral sinuses, and bulging of posterior fossa. The combination of these signs usually makes diagnosis easy. In nearly all cases it is confirmed by ventriculography and there have been only rare reports of a complete diagnosis being made by encephalography (Schreibner and Reye, 1954). In the case reported here, the diagnosis was revealed by lumbar encephalography in which air entered the characteristically enlarged 4th ventricle.
Female, born 4.12.68. Birth weight 3·5 kg. Head circumference 36·8 cm. Pregnancy was complicated by intermittent vomiting except for a short period where the mother took Promethazine Theoclate (Avomine) 25 mg, tabs 2 daily. There was possible contact with rubella in the fifth month. No other maternal infections. Labour induced one week after expected date of delivery, lasted for about 24 hours and was said to be normal as was the delivery. Respiration commenced within two minutes. At two days of age she was seen by a consultant paediatrician and wide anterior and posterior fontanelles were noted. Also present were microphthalmia, macroglossia, macrostomia and an extra digit on the right foot.
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